Search :     
Articles » Tumors » Neuroblastoma

2006-04-04-15 Neuroblastoma © Cuillier www.thefetus.net/


Neuroblastoma

Cuillier F, MD*, Carasset G, MD**, Lemaire P***, Deshayes M***

* Department of Gynecology ** Gynecologist, Saint Clothil clinic *** Sonogapher, Moufia’street , Hôpital Félix Guyon - Ile de la Réunion, France

Definition: Neuroblastoma is a sarcoma consisting of malignant neuroblasts, usually arising from cells of the autonomic nervous system (1/3) or the adrenal medulla (2/3). Neuroblastoma is the most common malignant tumor in infancy and early childhood (1).

Approximately:

  • 70% develop in the abdomen, with about half being in the adrenal gland
  • 20% occur in the thoracic cavity
  • 10% occur in all the areas where cells of the neural crest can be found.

The most frequent metastases are in the liver, bones, marrow and skeleton.
We report one case of fetal adrenal mass detected by routine prenatal ultrasound.

Synonyms: Neuroblastoma in situ ; Adrenoblastoma.

Case report: A 35-year-old primigravid woman had a normal 13 and 22 weeks scan. The nuchal translucency and the triple test were strictly normal. At 34 weeks, the patient underwent a routine ultrasound examination. The scan revealed a heterogeneous, hypoechoic and hyperechoic mass, between the upper pole of the right kidney and the diaphragm, measuring 29 x 30 x 32 mm (Figure N°1-2-3-4). The color Doppler demonstrated that the tumor was arising from the adrenal gland and there was a poor vascularization around the tumor (Figure N°5). Fetal biometry, biophysical profile and Doppler examination were normal (Figure N°6). At 34 weeks, a MRI was performed and confirmed the right heterogeneous mass (Figure N°9-10). A adrenal hemorrhage and a neuroblastoma were suspected. The mother did not demonstrate any adrenergic symptomatology or hypertension. Fetal biometry and morphology were otherwise normal. At 35, 37 and 39 weeks, another scans were performed (Figure N°7-8).

At 40 weeks, the baby was delivered (2800g) vaginally.  The Apgar score was 10/10 at 1/5 minutes, respectively. A small right flank mass was palpable on physical examination. Vanilmandelic acid and catecholamine levels were negative in a 24-hour-urine sample. MRI and ultrasound scans confirmed a right solid adrenal mass. Perfusion scan showed contrast enhancement and calcifications suggestive of neuroblastoma (Figure N°11-12).

Preoperative diagnostic evaluation was otherwise normal and included bone marrow aspirate, bone scan, urinary catecholamines and liver function tests. Concentrations of catecholamines metabolites and vanilmandelic acid were not elevated in the neonatal blood. Adrenal scintigraphy was done and confirmed the neuroblastoma diagnosis. A right tumorectomy was performed on day-25.  Pathological examination showed an encapsulated stage I neuroblastoma with favorable histology. No further treatment was performed. After one month, the baby is healthy.

Figure 1,2,3,4 :Transverse view at 34 weeks showing a hypoechoic suprarenal mass

Figure 5: Transverse view at 34 weeks showing a solid echogenic suprarenal mass
Figure 6: 3D of the fetal face

Figure 7: Parasagital view at 37 weeks showing normal left kidney and right normal kidney

Figure 8: Parasagital view at 39 weeks showing normal left kidney and right normal kidney

Figure 9: MRI at 34 weeks showing a hypoechoic suprarenal mass (right) and the right kidney (left).
Figure 10: MRI at 34 weeks showing the lesion appeared echoic and anechoic with multiple septa

Figure 11: Postnatal MRI view showing right heterogeneous mass located at the superior pole of the kidney
Figure 12: Postnatal coronal MRI showing a sagittal view of neuroblastoma with intracystic septations

History: Prenatal neuroblastoma diagnosis was first reported in 1983 by Fenart et al and after that more than 55 case have been published (2). The localization is essentially on adrenal. All published reports of prenatally diagnosed cases were detected in the third trimester of pregnancy. Recently De Filipi et al reported one case of a thoracic mass seen at 37 weeks. Atkinson et al detected a complex abdominal mass at 32 weeks although a previous ultrasound in the 16 weeks showed no pathology (4). Kesrouani et al (1999) described two cases of antenatal neuroblastoma. Both cases had favorable outcome and surgery was necessary in only one (1).

Prevalence: Neuroblastoma represents 30% of all neonatal tumors and it is the most common malignant tumor (6). The incidence of neuroblatoma ranges from1/10.000 to 1/30.000 in childhood according to Kessouani (1). This discrepancy is caused by a high rate of spontaneous regression that is well documented in infants younger than one year of age without treatment. According to Girschick (2), the incidence of congenital neuroblatoma had been estimated in 10.000-30.000 live births, but in area where screening for neuroblatoma during the first six months of life was introduced, the incidence was found to be two to three times higher than expected. This is consistent with autopsy series, which show that incidental neuroblstamo in situ occur in 1:200 to 1:250 among stillbirths and infants who have died under three months of age. Although neurobastoma is the most common abdominal malignancy diagnosed in neonates and early childhood, it is seen as frequently as 1/40 in autopsy studies (1/259 cases according to Beckwiths). This is 40 times greater than the incidence of clinically manifested (6).

  • Neonatal prevalence:  1,9 / 1000
  • Prenatal prevalence:  Unknown.
  • Neuroblastoma is slightly more frequent in the white population and in males.
  • About 2/3 of cases are located in the adrenal gland
  • The right side appears to be more frequently affected

Etiology: Most neuroblastomas are sporadic, although a small number may be familiar with complex inheritance patterns (7). Congenital neuroblastoma has also been reported following treatment with phenobarbital and phenytoin (coincidental association?). Adverse biologic features are diploid tumor karyotype (cytometry) and amplify neuroblastoma-myc oncogene. A genetic defect is involved in the etiology, with a loss of critical region in Chr 1 (locus p36). Amplification of the neurobastoma-myc proto-oncogene is also correlated with the aggressiveness of the tumor